Acute hemorrhagic edema (AHE) of childhood, a variant of Henoch-Schönlein purpura (HSP), is a rare vasculitis with benign course, generally no systemic involvement and rare flares. From January 1983 to June 2004, 4,502 patients were followed at the Pediatric Rheumatology Unit, Hospital of Clinics. Diagnosis of HSP was made in 203 cases (4.5%), of which 5 (0.1%) had AHE. All patients with AHE were male and the mean age at onset was 18 months (range: 8 to 21 months). All five cases presented vasculitis with characteristic hemorrhagic and purpuric lesions in malar region of the face, associated with painless edema of the hands and feet. Laboratory exams were normal. Upper respiratory tract infection preceding clinical manifestations occurred in four and mononucleosis in one. Treatment with corticosteroids was necessary only in one patient with necrotic lesions on the face and ears.